Pediatrics & Neonatology
Volume 49, Issue 4 , Pages 154-157, August 2008

Hydranencephaly in Neonates

  • Jeng-Dau Tsai

      Affiliations

    • Department of Pediatrics, Chung Shan Medical University Hospital, Taichung, Taiwan
    • Corresponding Author InformationCorresponding author. Department of Pediatrics, Chung Shan Medical University Hospital, 110 Chien-Kuo North Road, Section 1, Taichung 402, Taiwan
    • ,
  • Huang-Tsung Kuo

      Affiliations

    • Department of Pediatrics, China Medical University Hospital, Taichung, Taiwan
    • ,
  • I-Ching Chou

      Affiliations

    • Department of Pediatrics, China Medical University Hospital, Taichung, Taiwan

Received 5 May 2007; received in revised form 16 June 2008; accepted 28 August 2008.

Article Outline

Hydranencephaly is characterized by severe dysgenesis of the cerebral hemispheres, with relative preservation of the thalamus, cerebellum and brainstem. Modern neurological imaging techniques have demonstrated that the affected regions are usually localized within the territories of the middle cerebral arteries. Evoked potentials have confirmed the imaging findings. This article reports on two neonates with hydranencephaly who presented with normal appearance. Neurological images were characterized by marked reduction of the total brain cortex, which was replaced by fluid. Evoked potentials demonstrated preserved auditory evoked potentials but a total absence of visual evoked potentials. These diagnostic techniques provided efficient and precise information concerning the extent of intracranial dysgenesis.

KEY WORDS:  evoked potentials , hydranencephaly , neonates

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PII: S1875-9572(08)60032-7

doi:10.1016/S1875-9572(08)60032-7

Pediatrics & Neonatology
Volume 49, Issue 4 , Pages 154-157, August 2008

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